Erythrocyte aplasia and systemic lupus erythematosus

Abstract

Pure erythrocyte aplasia is a recognised feature of systemic lupus erythematosus (SLE); here we report two cases, one predating the onset of SLE, the other following a long period of disease quiescence. One case demonstrates the typical features of this disorder and was successfully treated with prednisolone. The second case is unusual in being resistant to immunosuppressive treatment. Bone marrow culture from the second patient revealed an inhibition of BFU-E colony formation in the presence of the patient's serum, indicating that a serum inhibitor of haemopoiesis was present. Furthermore, following T cell depletion of this patient's marrow, there was an increase in BFU-E, CFU-G and CFU-GM colony growth implicating, in addition, a possible T cell-mediated inhibition of marrow haemopoiesis. This is a novel observation and may explain the resistance shown by this patient to standard treatment.

Keywords

red cell aplasia-pure haemopoiesis cytokines

Get full access to this article

View all access options for this article.

References

Daughaday WH Lupus erythematosus with severe anaemia, selective erythroid hypoplasia and multiple red blood cell isoantibodies (CPC). Am J Med 1968; 44: 590-598.

Saxon A. , Feldhaus J. , Robins RA Single step separation of human T and B cells using AET treated srbc rosettes . J Immunol Methods 1976; 12: 285-288.

Sutherland HJ , Eaves AC , Eaves CJ Quantitative assays for human hematopoietic progenitor cells. In: Gee AP (ed.). Bone Marrow Processing and Purging: Practical Guide. Boca Raton: CRC press, 1991, pp 155-171.

Eaves AC , Eaves CJ Erythropoiesis in culture. Clin Hematol 1984 ; 13: 371-390.

McGuckin CP , Ball SE , Gordon-Smith EC Diamond-Blackfan anaemia: three patterns of in vitro response to haemopoietic growth factors. Br J Haematol 1995; 89: 457-464.

Franzen P. et al. Combined pure red cell aplasia and primary autoimmune hypothyroidism in systemic lupus erythematosus. Arthritis Rheum 1987; 30: 839-840.

Francis DA Pure red cell aplasia: association with systemic lupus erythematosus and primary autoimmune hypothyroidism. Br Med J 1982; 284: 85.

Heck LW et al. Pure red cell aplasia and protein losing enteropathy in a patient with systemic lupus erythematosus. Arthritis Rheum 1985; 28: 1059-1061.

MacKenzie Hln et al. Thymoma, myasthenia gravis, erythroblastopenic anaemia and systemic lupus erythematosus in one patient. Can Med Assoc J 1973; 109: 733-738.

10.

Cassileth PA , Myers AR Erythroid aplasia in systemic lupus erythematosus . Am J Med 1973; 55: 706-709.

11.

Krantz SB , Moore WH , Zaentz SD Studies on red cell aplasia. V. Presence of erythroblast cytotoxicity in γG-globulin fraction of plasma. J Clin Invest 1973; 52: 324-336.

12.

Nitsche A. et al. Pure red cell aplasia in a patient with systemic lupus erythematosus . J Rheumatol 1988; 15: 1012-1013.

13.

Peschle C. et al. Pure red cell aplasia: studies on an IgG serum inhibitor neutralizing erythropoietin. Br J Haematol 1975; 30: 411-417.

14.

Jepson JH , Lowenstein L. Panhypoplasia of the bone marrow. I. Demonstration of a plasma factor with anti-erythropoietin-like activity. Can Med Assoc J 1968; 99: 99-101.

15.

Cavalcant J. et al. Red cell hypoplasia and increased bone marrow reticulin in systemic lupus erythematosus: reversal with corticosteroid therapy. Am J Haematol 1978; 5: 253-263.

16.

Krantz SB , Kao V. Studies on red cell aplasia. I. Demonstration of a plasma inhibitor to heme synthesis and an antibody to erythroblast nuclei. Proc Natl Acad Sci USA 1967; 58: 493-500.

17.

Perdahl EB et al. Erythroid failure in Diamond-Blackfan anaemia is characterized by apoptosis. Blood 1994; 83: 645-650.

18.

Dessypris EN Pure red cell aplasia. In: Shahidi NT (ed.). Aplastic Anaemia and Other Bone Marrow Failure Syndromes. Springer: Berlin, 1990, pp 179-198.

19.

Krantz SB Studies on red cell aplasia. III. Treatment with horse antihuman thymocyte gamma globulin. Blood 1972; 39: 347-360.

20.

Ishiyama T. et al. Spontaneous IL-2 production in vitro in two patients with pure red cell aplasia. Ann Haematol 1993; 67: 249-252.